Anormalidades Pulmonares Vascular

8/19/2019 Anormalidades Pulmonares Vascular

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Pulmonary vascular anomalies in adult; a pictorial review

Award: Magna Cum Laude

Poster No.: C-0901

Congress: ECR 2015

Type: Educational Exhibit

Authors: K. Tokunaga, T. Yamaoka, A. Hamada, T. Kubo, K. Togashi;Kyoto/JP

Keywords: Lung, Pulmonary vessels, CT, CT-Angiography, ComputerApplications-3D, Developmental disease, Arteriovenousmalformations, Dysplasias

DOI: 10.1594/ecr2015/C-0901

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Learning objectives

1. To illustrate characteristic CT findings of pulmonary vascular developmental

anomalies seen in adult cases.

2. To clarify differential key points of pulmonary vascular anomalies.

3. To make a concise review of the embryological basis of pulmonary vascular

anomaly to facilitate deeper understanding of pathophysiology.

Background

There are number of pulmonary vascular developmental anomalies. They can be

diagnosed because of symptoms/complications or discovered incidentally without any

symptoms. And from operation to observation, there is wide variety of therapeutic options.

Reasonable choice of therapeutic options depends on correct diagnosis.

Findings and procedure details

1. Normal development of the pulmonary system;

1.1. Lung and Bronchi

Development of the lung starts from respiratory diverticulum, a bud of the primitive

foregut, at 3 weeks of gestation. It repeats asymmetrical branching to form the

tracheobronchial tree and the lung parenchyma throughout five stages, embryonic,

pseudoglandular, canalicular, saccular and alveolar stages [1]. Respectively, each part

of the respiratory system develops gradually by the gestational age. Trachea and the

main bronchi appear at 3 to 7 weeks of gestation, the terminal bronchi at 5 to 17 weeks,

and the respiratory bronchiole at 16 to 26 weeks. The lung development continues after

birth, and completes at 2 to 3 years of age [1].

Peripheral pulmonary vasculatures develop independently and simultaneously from

central pulmonary vasculature. They connect at several points in the mid fatal stage.

Branches from the pulmonary venous system connect to the capillary network at 12 to

14 weeks of gestation. Vascularization of the terminal airways occur at 16 to 26 weeks,capillary network closely approaches the airway epithelium, which is accompanied with

the pulmonary venous branch, connected at 12 to 14 weeks, and pulmonary artery

branch, establishes connection at 22 to 23 weeks [1].


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Fig. 1: Normal development of the lung.References: - Kyoto/JP

1.2. Pulmonary Vascular System

1.2.1. Pulmonary Artery system

There are six primitive aortic arches in the embryogenesis. They bridge the central

structures (aortic sac and ventral aorta) with dorsal aortas of both sides. The proximal

(extrapulmonary) portion of the pulmonary artery develops mainly from the proximal

portion of the sixth primitive aortic arches. The distal segment of left sixth aortic arch

remains as the ductus arteriosus, while on the right side, the distal portion of the

primitive arch involutes. The aortic sac develops into main pulmonary artery and thoracic

ascending aorta [2].


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Fig. 2: Normal development of the primitive aortic arches.References: - Kyoto/JP

1.2.2. Pulmonary Venous System

During early embryogenesis, the pulmonary venous plexus is closely associated with

the splanchnic plexus. Immature pulmonary venous drainage from the lung buds is

via the splanchnic plexus into the cardinal and umbilicovitelline venous plexus. Right

cardinal venous plexus develops into the superior vena cava (SVC), while left one mostly

disappears. Umbilicovitelline venous plexus develop into the inferior vena cava (IVC),

portal venous system, and ductus venosus. There is no direct drainage into the heart at

this stage.

By day 27-28 of gestation, an outpouching from the primitive atrial wall arises toward thecommon venous plexus to form the common pulmonary vein. By day 30, the pulmonary

venous plexus has mostly separated from the splanchnic, cardinal, and umbilicovitelline

venous plexuses. The common pulmonary vein is absorbed into the dorsal wall of the

growing left atrium, leaving four pulmonary veins separately draining into the left atrium

[2].


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Fig. 3: Normal development of the venous plexuses and pulmonary venous system.References: - Kyoto/JP

2. Pulmonary vascular developmental abnormality;

2.1 (Isolated) Unilateral Absence of a Pulmonary Artery (UAPA)

UAPA is characterized by absence of one of the pulmonary arteries. Estimated

prevalence is 1 in 200,000 young adults [3]. Right sided is more common [3]. This may

occur in an isolated manner [3] or may associate with congenital heart disease such as

tetralogy of Fallot or septal defects [3]. UAPA is caused by involution of the proximal

segment of sixth aortic arch [3]. So, rudimentary pulmonary arteries can be identified

pathologically in the lung. Symptoms include those related with pulmonary hypertension

(dyspnea on effort) and excessive systemic arterial supply (hemoptysis). However, 30%of the cases are asymptomatic [3].

Radiologically, the most characteristic finding is abrupt interruption of the proximal portion

of the pulmonary artery. Intrapulmonary vessels of the affected side are smaller than the

contralateral. Prominent collateral arteries from systemic circulation can be visualized


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on 3D-CT angiography. Mosaic attenuation of lung parenchyma, interlobular septal

thickening, and cylindrical bronchiectasis are commonly demonstrated [4].

Ventilation-perfusion mismatch is a useful finding for differentiating UAPA from Swyer-

James Syndrome.

Therapeutic option include 1) medications or revascularization for pulmonary

hypertension [3], 2) embolization for moderate hemoptysis [3], and 3) surgical resection

of the affected lung [3] for massive hemoptysis.

Fig. 4: Right UAPA in a 62-year-old female patient with massive hemoptysis(case 1): chest X-ray presents displacement of the heart, trachea andmediastinum to the right, volume loss of the right lung, small right hilum and thepulmonary vessels, infiltration of the right middle lung field.References: - Kyoto/JP


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Fig. 5: Contrast enhanced chest CT and 3D-CT angiography of the case 1:demonstrates total absent of the right pulmonary artery from its origin. Lung-windowCT image demonstrates volume loss of the right lung, ground glass opacity, and

emphysematous change of bilateral lung. CT angiography also shows collateral arterydevelopment from bronchial, intercostal, intrathoracic, inferior phrenic, and coronaryarteries to the right lung. Narrowed right pulmonary vein: complex anastomoses amongcollateral systemic arteries are also demonstrated.References: - Kyoto/JP


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Fig. 6: 3D-CT angiography of the case 1: demonstrates collateral artery developmentfrom bronchial, intercostal, intrathoracic, inferior phrenic, and coronary arteries to theright lung.References:

- Kyoto/JP


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Fig. 7: Ventilation-perfusion lung scan of the case 1: demonstrates V/Q mismatch ofthe total right lung field (ventilation uptake ratio in the right lung was decreased to 50%of the left side).References:

- Kyoto/JP


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Fig. 8: Histological findings of the surgical specimen of case 1: small and collapsedelastic artery is seen only at the proximal part, suspected as rudimentary pulmonaryartery (arrow). Around the elastic artery and bronchi in the interlobular septa, dilated

and meandering muscular arteries are seen, suspected as bronchial artery itself orarising collateral artery (arrow head). Br: bronchi, LN: lymph nodes, PV: pulmonaryvein (tissue marking dyestuff was injected to the pulmonary vein before formalinfixation) (Elastica van Gielson stain).References: - Kyoto/JP


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Fig. 9: Right UAPA in a 67-year-old male patient with dyspnea (case 2): chests X-raypresents displacement of the heart, trachea and mediastinum to the right, volume lossof the right lung, small right hilum, and diffuse ground glass opacity.References:

- Kyoto/JP


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Fig. 10: Contrast enhanced chest CT and 3D-CT angiography of the case 2:demonstrates proximal interruption of the right pulmonary artery. Lung-window CTimage shows cystic bronchiectasis.References:

- Kyoto/JP

2.2 Idiopathic Pulmonary Artery Aneurysm (PAA)

Pulmonary artery aneurysm is characterized by aneurysmal dilation of the pulmonary

artery secondary to disintegration of its wall [5]. The endogenic weakness of the arterial

wall with increased hemodynamic stress seems to be responsible for its formation [6].

Estimated prevalence is 1 in 14,000 autopsies [6]. Pathological criteria for idiopathic PAA

are described as 1) dilation of pulmonary trunk (involvement of arterial tree might or might

not be present), 2) absence of extra- or intracardiac shunts, 3) absence of pulmonary

disease or chronic cardiac disease, and moreover, 4) minimal atheromatosis, pulmonaryvascular tree arteriosclerosis or absence of arterial disease [7]. Rupture and dissection

are fatal complication of PAA. Most patients are asymptomatic.

Radiologically, saccular or fusiform dilation of the pulmonary artery in various sizes [6] is

demonstrated. PAA show homogeneous enhancement pattern to the pulmonary artery.


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It is very difficult to differentiate from other acquired aneurysms (Takayasu's arteritis,

Williams syndrome, prenatal varicella, Behcet's syndrome etc.) on imaging only [8].

Surgical intervention is recommended in large sized or pulmonary regurgitated case [6]

because of its high morbidity. Conservative treatment is advocated in mild idiopathic

cases [6].

Fig. 11: Idiopathic PAA (A9) in a 62-year-old female patient, pointed out on healthscreening chest X-ray (case 3): chest X-ray shows a nodule (arrow) in the proximalarea of the right lower lung field.References: - Kyoto/JP


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Fig. 12: Oblique coronal MIP image of contrast enhanced chest CT and 3D-CTangiography of the case 3: demonstrates a focal arterial enlargement of the proximalportion of right A9. PAA shows homogeneous enhancement pattern to the pulmonary

artery.References: - Kyoto/JP

2.3 Pulmonary Sequestration

Pulmonary sequestration is characterized by a non-functioning mass of normal lung

tissue without normal tracheobronchial communication and receiving blood supply from

the systemic circulation. Estimated prevalence is 0.15-1.8% in pediatric study [9]. The

left lower lobe is the most common site [9]. They are classified into 2 groups based on

their pleural coverage;

1) Extralobar pulmonary sequestrations (EPS); masses of lung parenchyma which is

separated individually with proper pleural coverage. Venous drainage into the systemic

vein is usual, however, in about 25% drains completely or partially into the pulmonary

vein. 65% of patients have associated congenital anomalies. Most of EPS is seen in

infants.


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2) Intralobar pulmonary sequestrations (IPS); masses of lung parenchyma without

proper pleural coverage. They are contiguous with the normal lung. Most of the drainage

vein runs into the pulmonary vein. Most of PS seen in adults is IPS type.

Embryogenesis of PS is controversial. Development of an accessory lung bud from the

primitive foregut [9] may be an embryological cause of PS. The accessory lung bud

receives its blood supply from the systemic circulation. These connections with aorta

remain as the systemic arterial blood supply to the sequestration. Development of the

accessory lung bud during early embryologic stage results in the intrapulmonary type,

and during later period, results in the extrapulmonary type, respectively [9].

EPS is often asymptomatic at birth, and develops congestive cardiac failure, respiratory

distress, or feeding difficulties [9] in infancy. On the other hand, IPS rarely causes

problems before the age of two years [9]. It may show chronic or recurrent pulmonary

infection, high output cardiac failure, hemoptysis or massive hemothorax in adult [9].

Radiologically, it shows a complex mass with/without cystic/cavitary changes and

emphysematous changes around the PS [9]. It is a key to suspect PS to identify the

systemic arterial supply to the mass. Differential diagnosis includes congenital cysticadenomatoid malformation (CCAM) [9] for EPS, and organizing pneumonia or lung

abscess for IPS [9]. Anomalous systemic arterial supply to the basal lung is one of

differential diagnosis on 3D-CT angiography, too.

Observation is possible in asymptomatic cases. Surgical resection is considered for

symptomatic cases. Embolization of the feeding artery could be an option when cardiac

decompensation occurs [9].


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Fig. 13: Left IPS in a 39-year-old male patient, referred for further examination ofabnormal mass shadow on health screening chest X-ray (case 4): chest X-ray presentsan irregular shaped mass in medial aspect of left lung base, an arc-shaped tubular

shadow between the left hilum and the mass.References: - Kyoto/JP


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Fig. 14: Contrast enhanced chest CT and 3D-CT angiography of the case 4;demonstrates emphysematous change in the left lung base. Bronchial structuresexists, however, they have no connection with normal bronchial tree. Two feeding

arteries arise from the thoracic descending aorta. Venous drainage runs into the leftinferior pulmonary vein. Boarder between the normal lung parenchyma and the lesionis unclear, mild consolidation suspected to be due to past pneumonia.References: - Kyoto/JP

2.4 Anomalous Systemic Arterial Supply to the Basal Lung

Anomalous systemic arterial supply to the basal lung is characterized by an anomalous

systemic arterial supply to the basal segments of the lung without pulmonary arterial

supply to the lung base [10]. This anomaly can be considered as a result of connection

failure between primitive extrapulmonary pulmonary artery and the plexiform peripheralpulmonary artery followed by persistent connection between primitive aortic branches

[10]. Normal tracheobronchial connection can be identified in the affected lung base. Left

side is more common. Patients are mostly asymptomatic, but may have hemoptysis and

exertional dyspnea [10].


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Radiologically, it is important to identify retrocardiac structure arising from the descending

aorta to the basal segments where normal lung parenchyma, pleura, and normal

bronchial structures are identified [4]. Posterobasal bronchi without accompanied

homonymous pulmonary artery may be seen [4]. Bronchial connections are the clue to

differentiate from PS.

Treatment is mandatory for all cases even asymptomatic, for its poor prognosis

from gross hemoptysis and heart failure [10]. Several therapeutic options including

anastomosis of the divided anomalous systemic artery to the pulmonary artery [10],

simple ligation of the anomalous artery [10], or coil embolization [10].

Fig. 15: Left anomalous systemic arterial supply to the basal lung in a 77-year-old female patient with hemoptysis and recurrent pneumonia (case 5); chest X-ray

presents cardiomegaly and retrocardiac shadow.References: - Kyoto/JP


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Fig. 16: Contrast enhanced chest CT and 3D-CT angiography of the case 5;demonstrates pneumonic consolidation in the left lower lobe, multiple focal groundglass appearance due to hemoptysis, and abnormal artery. The abnormal artery arises

from the thoracic descending aorta running into medial part of the left lower lobe, withnormal bronchial connection.References: - Kyoto/JP

2.5 Pulmonary Arteriovenous Malformation (pAVM)

Pulmonary arteriovenous malformation (pAVM) is an abnormal communication between

the pulmonary artery and vein. Estimated prevalence is 2-3 per 100,000 populations

[11]. Approximately 47-80% of pAVMs are associated with hereditary hemorrhage

teleangiectasia (HHT) or Osler-Render-Weber disease [11], and about 15-30% of HHT

patients have more than one pAVM(#6). More than 50% of pAVMs are located in thelower lobes.

Normally vascular septa divide the primitive connections between the arterial and venous

plexuses. PAVM is considered as a result from disintegration of vascular septa [5].

Specific gene alterations are known [11]; such as HHT3 (5q31.3-q32), HHT4 (7p14),

BMPR2 (2q33.1), OWR-1 (9q 33-34), OWR-2 (12q) or (3q22) [5,11]. Acquired causes


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such as liver cirrhosis, hepatopulmonary syndrome, schistosomiasis, mitral stenosis,

trauma, actinomycosis, metastatic thyroid carcinoma, systemic amyloidosis, and chronic

inflammatory condition such as bronchiectasis may also develop pAVM.

Approximately 13-55% cases are asymptomatic [11], particularly in case of solitary

pAVM smaller than 2cm in diameter. The most common complications is paradoxical

embolism across the pAVM [11]. Less common but life threatening complications include

hemoptysis and hemothorax due to rupture of AVM [11].

Radiologically, pAVM is a homogeneous, well-demarcated, a non-calcified nodule up

to several centimeters in diameter with connecting to both pulmonary artery and vein.

Simultaneous enhancement of the feeding pulmonary artery, the aneurysmal nidus, and

early venous return to the draining pulmonary vein maybe demonstrated on pulmonary

artery phase of dynamic CT. Differential diagnosis of feeding vessel sign includes septic

pulmonary embolism, pulmonary metastases, hemorrhagic nodules, or consolidation

seen in vasculitis [12]. However, these differential diagnoses may have no connection

to the draining pulmonary veins.

To prevent neurological complications, progressive hypoxia, and high output cardiacfailure, when there are any symptom, large pAVM larger than 2cm, or feeding artery

greater than 3mm, surgical resection or embolization is recommended [11].


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Fig. 17: Left pAVM in a 71-year-old female patient, pointed out on pre-operativescreening chest X-ray (case 6); chest X-ray shows a nodular shadow in the left middlelung field (arrow).References:

- Kyoto/JP


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Fig. 18: Contrast enhanced chest CT and 3D-CT angiography of the case 6;demonstrates string of beads like shadow in the left lower lobe (S6). The nodulesare mildly contrast enhanced. Paired dilated vessels from hilum to the nodule are the

feeding pulmonary artery and draining pulmonary vein.References: - Kyoto/JP


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Fig. 19: Right pAVM in a 68-year-old female patient with hemoptysis (case 7); chestX-ray presents ground glass opacities at the right middle lung field, mild dilated tubularshadow running from right hilum to the basal lung field (arrow).References:

- Kyoto/JP


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Fig. 20: Contrast enhanced chest CT and MIP image of the case 7; demonstratestubular structure at the right lower lobe. Paired dilated vessels running directly fromhilum to the nodule are the feeding artery (red arrow) and draining vein (blue arrow).

PA: feeding artery from the pulmonary artery, PV: draining vein to the pulmonary vein.References: - Kyoto/JP

2.6 Partial Anomalous Pulmonary Venous Return (PAPVR)

PAPVR is a condition at least one of the pulmonary veins drains into other structure than

the left atrium. Estimated prevalence is 0.4-0.7%. Most cases are sporadic, but genetic

factors have been suggested in some familial cases [13]. Right sided is more frequently

affected than the left, although left sided PAPVR is more easily detectable on CT for its

draining course. The anomalous connection can be classified into four groups based on

their drainage [13]. 1) Supracardiac (to SVC, azygos vein, vertical vein, left SVC, etc.),2) cardiac (right atrium or coronary sinus), 3) infradiaphragmatic (IVC, portal vein, etc.),

and 4) mixed.

There are 2 dominant explanations. Hypothesis 1: misconnection between common

pulmonary vein to the splanchnic plexus with the remaining anastomosis. Bronchial veins

could play a key role in the occurrence and course of PAPVR [13]. Hypothesis 2: shift of


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the common pulmonary vein with regard to the atrial septum during rotation of the sinus

venosus to the right may result in part of the common pulmonary vein being incorporated

into the right side of the primitive atrium.

They are most commonly asymptomatic and may be incidentally detected on either CT

or MRI. In symptomatic cases, symptoms are generally absent in childhood, may present

later in life with congestive heart failure. Symptoms become clinically significant when

50% or more of the pulmonary blood flow returns anomalously.

CT shows pulmonary venous connection to the systemic vein. Right heart enlargement

with septal defect, enlarged central pulmonary arteries, pulmonary venous congestion

may be demonstrated [13]. Differential diagnosis includes total anomalous pulmonary

venous return (TAPVR), pulmonary varix. Persistent left SVC looks like left PAPVR on

CT.

Surgical repair is the definite therapy for symptomatic cases [13].

Fig. 21: Left PAPVR in a 57-year-old male patient, pointed out on pre-operativescreening chest X-ray (case 8); contrast enhanced chest CT and MIP image


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demonstrates the left superior pulmonary vein drains into the left brachiocephalic vein(arrow).References: - Kyoto/JP

Fig. 22: Right PAPVR in a 69-year-old female patient, pointed out on pre-operativechest CT for lung cancer in the right lower lobe (case 9); contrast enhanced chest CTand 3D-CT angiography reveals the right superior pulmonary vein draining into thesuperior vena cava. Rudimentary small right pulmonary vein (V2), dorsal to the rightpulmonary artery, drain into the left atrium.References: - Kyoto/JP

2.7 Anomalous Unilateral Single Pulmonary Vein (AUSPV)

AUSPV is characterized by the absence of one of the pulmonary veins and therefore

the entire ipsilateral lung flow drains into the left atrium via the meandering dilated singlepulmonary vein. The right lung is the more commonly involved. Frequently associate

with hypoplastic right lung and dextrocardia. Infrequently, a small secondary vein may

drain into a systemic vein in the chest that flows into right atrium. AUSPV is usually

asymptomatic. No treatment is required for AUSPV [14].


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AUSPV is considered to occur as a consequence of atresia or hypoplasia of one of

the pulmonary veins prior to the segmentation and the incorporation failure of common

pulmonary vein to properly into the dorsal left atrial wall, with the entire lung subsequently

draining via the remaining vein [14]. Gene mutations in the morphogenetic protein

receptor II gene (BMPR2) and wild-type activin receptor like kinase 1 (ALK1) was reported

[15].

CT demonstrates absent relevant lobar pulmonary vein and abnormal dilated (varicose)

pulmonary veins. Abnormal vessels taking tortuous and detoured course with crossing a

lung fissure before draining into the ipsilateral pulmonary vein [14]. Differential diagnoses

include scimitar syndrome, a subtype of PAPVR, and pAVM. AUSPV can be differentiated

by evaluating continuity of the pulmonary vessels.

Fig. 23: Right AUSPV in a 55-year-old female patient, pointed out on health screeningchest X-ray (case 10); chest X-ray demonstrates a nodule like pAVM at the left lowerlung field (arrow).References: - Kyoto/JP


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Fig. 24: Sectional CT and 3D-CT angiography of the case 10; demonstrates twoabnormally dilated vessels. Both vessels continue to the left inferior pulmonary vein,and the left superior pulmonary vein is absent.References:

- Kyoto/JP

2.8 Pulmonary Angiectopia with Central Pulmonary Venous Aplasia (Congenital

Pulmonary Vein Stenosis and Hypoplasia/Atresia)

Congenital pulmonary vein stenosis and hypoplasia/atresia reflects a spectrum of the

same abnormality. They are characterized by narrowing or occlusion of one or more

pulmonary veins at their junction with the left atrium, or at individual pulmonary veins

[16]. Estimated prevalence is 1.7 per 100000 children (< 2 years old) [16]. Abnormality

may be solitary or multiple, may be focal or involved long segment of pulmonary vein.

Approximately 50% of affected patients are associated with congenital heart disease [16].

Congenital cases are suspected to result from the defective abnormal incorporation of the

common pulmonary trunks into the left atrium in the later stages of cardiac development

[16]. Acquired cases are may be due to constructive pericarditis, mediastinitis,

tuberculosis, obstructive tumors or operative scar [16].


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Symptoms range from asymptomatic to severe. The presentation in the early stage

includes tachypnea, dyspnea, recurrent pneumonia and hemoptysis. As the disease

progresses, pulmonary hypertension, right heart failure and pulmonary edema are seen

[16].

Radiological findings include partial absence of pulmonary venous drainage into the

left atrium with the narrowed pulmonary vein in the affected lung, and pulmonary

parenchyma alterations such as interlobular septal thickening, peribronchovascular

thickening, interstitial fibrosis, and ground glass opacities [16]. Differential diagnosis

includes pulmonary veno-occlusive disease (PVOD). PVOD is a condition related with

primary pulmonary hypertension. No pulmonary vein stenosis/hypoplasia/atresia is seen

in PVOD.

Therapeutic options range from follow up to embolization, or surgical treatment

depending on presentations [16].

Fig. 25: Right congenital pulmonary vein atresia in a 53-year-old female patient,pointed out on health screening chest X-ray (case 11): chest X-ray demonstrated twonodules at the right upper lung field (arrow).References: - Kyoto/JP


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Fig. 26: MIP image and 3D-CT angiography of the case 11; demonstrates twoabnormally dilated vessels with homogeneous enhancement pattern to the pulmonaryveins. The vessels take detoured course of the pulmonary vein to drain into the left

atrium via the right superior pulmonary vein. On the other hand, proximal end ofapical pulmonary vein (V1) occludes nearby the right bronchus, but there is no absentpulmonary vein.References: - Kyoto/JP

2.9 Pulmonary Varix

Pulmonary varix is a condition consisting of abnormal dilatation of a segment of the

pulmonary vein. The most common site is right lower lobe. They are classified into three

groups according to its configuration: saccular, tortuous, and confluent. This could occur

congenitally, however, acquired form (mitral valve disease, etc.) are common [17]. Theetiology of congenital form is unknown, however, they are considered to develop as a

collateral pathway of a pulmonary venous stenosis/atresia [18] in embryonic stage [17].

They are usually asymptomatic. Rarely, complications such as systemic emboli caused

by the release of clots from within the varix or, even more infrequently, hemoptysis or

hemothorax caused by rupture of the varix may occur [5].


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Radiologically, saccular or tubular dilatation of the affected pulmonary vein which has

direct continuity with the left atrium is demonstrated [18]. Pulmonary venous connection

remains normal in most cases [18]. Bartram presented five angiographic criteria [17]: 1)

the arterial phase is normal without shunting, 2) the varix fills in the venous phase, 3) the

varix drains directly into the left atrium, 4) emptying of the varix is delayed, and 5) the

varix affects only the proximal portion of the vein.

Treatment is not indicated for most cases. If they develop any symptoms or complications

[17], embolization or surgical resection is considered [5].

Fig. 27: Right pulmonary varix in a 60-year-old male patient (case 12); MIPreconstruction images and 3D-CT angiography of contrast enhanced chest CT revealfocal dilation of the pulmonary vein (V1) at the proximal part of the right upper lobe

(arrow). Both proximal and distal pulmonary venous structures are within normal range,draining into left atrium through the normal right superior pulmonary vein.References: - Kyoto/JP

2.10 Primary racemose hemangioma of the bronchial artery


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Primary racemose hemangioma of the bronchial artery is characterized by enlarged

and convoluted bronchial arteries without any inflammatory changes or causes in the

pulmonary system [19]. Common site is the right lower lobe bronchi. More than half of

the cases show bronchial-pulmonary arterial shunt [20]. For diagnosis, bronchoscopic

examination or bronchial arteriography is essential. Primary racemose hemangioma of

the bronchial artery is considered to occur as an abnormal capillary growth through the

pulmonary system development, based on some sort of factors to increase capillary blood

flow [20]. Recurrent hemoptysis is often seen.

Radiologically, contrast enhanced study presents dilated bronchial arteries,

vascularization with remarkable dilation and convolution of vessels. Bronchial-pulmonary

arterial shunt is often seen [19].

Combination of embolization and surgical resection is performed as standard therapy.

Fig. 28: Racemose hemangioma of the right bronchial artery in a 39-year-old malepatient with recurrent hemoptysis (case 13): lung-window CT images demonstrate focalground glass opacities in the right upper lobe due to hemoptysis. Contrast enhancedchest CT and 3D-CT angiography demonstrates dilated and convoluted right bronchialarteries, although, no signs of primary factor in the lung, bronchi, or mediastinum.


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References: - Kyoto/JP

Images for this section:

Fig. 1: Normal development of the lung.


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Fig. 2: Normal development of the primitive aortic arches.


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Fig. 3: Normal development of the venous plexuses and pulmonary venous system.


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Fig. 4: Right UAPA in a 62-year-old female patient with massive hemoptysis (case 1):

chest X-ray presents displacement of the heart, trachea and mediastinum to the right,

volume loss of the right lung, small right hilum and the pulmonary vessels, infiltration of

the right middle lung field.


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Fig. 5: Contrast enhanced chest CT and 3D-CT angiography of the case 1: demonstrates

total absent of the right pulmonary artery from its origin. Lung-window CT image

demonstrates volume loss of the right lung, ground glass opacity, and emphysematous

change of bilateral lung. CT angiography also shows collateral artery development frombronchial, intercostal, intrathoracic, inferior phrenic, and coronary arteries to the right

lung. Narrowed right pulmonary vein: complex anastomoses among collateral systemic

arteries are also demonstrated.


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Fig. 6: 3D-CT angiography of the case 1: demonstrates collateral artery development

from bronchial, intercostal, intrathoracic, inferior phrenic, and coronary arteries to the

right lung.


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Fig. 7: Ventilation-perfusion lung scan of the case 1: demonstrates V/Q mismatch of the

total right lung field (ventilation uptake ratio in the right lung was decreased to 50% of

the left side).


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Fig. 8: Histological findings of the surgical specimen of case 1: small and collapsed

elastic artery is seen only at the proximal part, suspected as rudimentary pulmonary

artery (arrow). Around the elastic artery and bronchi in the interlobular septa, dilated and

meandering muscular arteries are seen, suspected as bronchial artery itself or arisingcollateral artery (arrow head). Br: bronchi, LN: lymph nodes, PV: pulmonary vein (tissue

marking dyestuff was injected to the pulmonary vein before formalin fixation) (Elastica

van Gielson stain).


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Fig. 9: Right UAPA in a 67-year-old male patient with dyspnea (case 2): chests X-ray

presents displacement of the heart, trachea and mediastinum to the right, volume loss of

the right lung, small right hilum, and diffuse ground glass opacity.


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Fig. 10: Contrast enhanced chest CT and 3D-CT angiography of the case 2:

demonstrates proximal interruption of the right pulmonary artery. Lung-window CT image

shows cystic bronchiectasis.


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Fig. 11: Idiopathic PAA (A9) in a 62-year-old female patient, pointed out on health

screening chest X-ray (case 3): chest X-ray shows a nodule (arrow) in the proximal area

of the right lower lung field.


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Fig. 12: Oblique coronal MIP image of contrast enhanced chest CT and 3D-CT

angiography of the case 3: demonstrates a focal arterial enlargement of the proximal

portion of right A9. PAA shows homogeneous enhancement pattern to the pulmonary

artery.


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Fig. 13: Left IPS in a 39-year-old male patient, referred for further examination of

abnormal mass shadow on health screening chest X-ray (case 4): chest X-ray presents

an irregular shaped mass in medial aspect of left lung base, an arc-shaped tubular

shadow between the left hilum and the mass.


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Fig. 14: Contrast enhanced chest CT and 3D-CT angiography of the case 4;

demonstrates emphysematous change in the left lung base. Bronchial structures exists,

however, they have no connection with normal bronchial tree. Two feeding arteries arise

from the thoracic descending aorta. Venous drainage runs into the left inferior pulmonaryvein. Boarder between the normal lung parenchyma and the lesion is unclear, mild

consolidation suspected to be due to past pneumonia.


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Fig. 15: Left anomalous systemic arterial supply to the basal lung in a 77-year-old

female patient with hemoptysis and recurrent pneumonia (case 5); chest X-ray presents

cardiomegaly and retrocardiac shadow.


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demonstrates pneumonic consolidation in the left lower lobe, multiple focal ground glass

appearance due to hemoptysis, and abnormal artery. The abnormal artery arises from

the thoracic descending aorta running into medial part of the left lower lobe, with normalbronchial connection.


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Fig. 17: Left pAVM in a 71-year-old female patient, pointed out on pre-operative

screening chest X-ray (case 6); chest X-ray shows a nodular shadow in the left middle

lung field (arrow).


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demonstrates string of beads like shadow in the left lower lobe (S6). The nodules are

mildly contrast enhanced. Paired dilated vessels from hilum to the nodule are the feeding

pulmonary artery and draining pulmonary vein.


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Fig. 19: Right pAVM in a 68-year-old female patient with hemoptysis (case 7); chest X-

ray presents ground glass opacities at the right middle lung field, mild dilated tubular

shadow running from right hilum to the basal lung field (arrow).


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Fig. 20: Contrast enhanced chest CT and MIP image of the case 7; demonstrates tubular

structure at the right lower lobe. Paired dilated vessels running directly from hilum to the

nodule are the feeding artery (red arrow) and draining vein (blue arrow). PA: feeding

artery from the pulmonary artery, PV: draining vein to the pulmonary vein.


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Fig. 21: Left PAPVR in a 57-year-old male patient, pointed out on pre-operative screening

chest X-ray (case 8); contrast enhanced chest CT and MIP image demonstrates the left

superior pulmonary vein drains into the left brachiocephalic vein (arrow).


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Fig. 22: Right PAPVR in a 69-year-old female patient, pointed out on pre-operative chest

CT for lung cancer in the right lower lobe (case 9); contrast enhanced chest CT and 3D-

CT angiography reveals the right superior pulmonary vein draining into the superior vena

cava. Rudimentary small right pulmonary vein (V2), dorsal to the right pulmonary artery,drain into the left atrium.


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Fig. 23: Right AUSPV in a 55-year-old female patient, pointed out on health screening

chest X-ray (case 10); chest X-ray demonstrates a nodule like pAVM at the left lower

lung field (arrow).


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Fig. 24: Sectional CT and 3D-CT angiography of the case 10; demonstrates two

abnormally dilated vessels. Both vessels continue to the left inferior pulmonary vein, and

the left superior pulmonary vein is absent.


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Fig. 25: Right congenital pulmonary vein atresia in a 53-year-old female patient, pointed

out on health screening chest X-ray (case 11): chest X-ray demonstrated two nodules at

the right upper lung field (arrow).


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Fig. 26: MIP image and 3D-CT angiography of the case 11; demonstrates two abnormally

dilated vessels with homogeneous enhancement pattern to the pulmonary veins. The

vessels take detoured course of the pulmonary vein to drain into the left atrium via the

right superior pulmonary vein. On the other hand, proximal end of apical pulmonary vein(V1) occludes nearby the right bronchus, but there is no absent pulmonary vein.


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Fig. 27: Right pulmonary varix in a 60-year-old male patient (case 12); MIP reconstruction

images and 3D-CT angiography of contrast enhanced chest CT reveal focal dilation of

the pulmonary vein (V1) at the proximal part of the right upper lobe (arrow). Both proximal

and distal pulmonary venous structures are within normal range, draining into left atriumthrough the normal right superior pulmonary vein.


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Fig. 28: Racemose hemangioma of the right bronchial artery in a 39-year-old male patient

with recurrent hemoptysis (case 13): lung-window CT images demonstrate focal ground

glass opacities in the right upper lobe due to hemoptysis. Contrast enhanced chest CT

and 3D-CT angiography demonstrates dilated and convoluted right bronchial arteries,although, no signs of primary factor in the lung, bronchi, or mediastinum.


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Conclusion

Pulmonary vascular anomalies accurately show non-specific symptoms and similar chest

X-ray presentations with some diseases. However, they show several characteristic

appearances and signs on CT based on their embryogenesis.

CT and/or 3D-CT angiography are a powerful tool to analyze pulmonary vascularanatomy and be helpful for appropriate management.

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Anormalidades Pulmonares Vascular

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